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Prenatal diagnosed cyst of the quadrigeminal cistern in Aicardi syndrome

Child's Nervous System volume 25, pages 521–522 (2009)Cite this article

Abstract

Aicardi syndrome is an X-linked dominant disorder characterized by the pathognonomic association of three clinical signs: agenesis of the corpus callosum, infantile spasms, and chorioretinal lacunae. This is a severe syndrome with an estimated survival rate of 76% at 6 years and 40% at 14 years and a maximum developmental level like a 12-month-old baby in 91% of cases. We illustrate a case of Aicardi syndrome with bilateral, small chorioretinal lacunae, infantile spasms with a typical hypsarrhythmia, defects of thoracic vertebra and bilateral absence of the12th rib associated with an arachnoid cyst of the quadrigeminal cistern, and presence of the corpus callosum with a favorable outcome.

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Affiliations

  1. Department of Neurosurgery Hannover, International Neuroscience Institute, Rudolf Pichlmayr-Str 4, 30625, Hannover, Germany

    Laura Columbano, Wolf Luedemann, Yasuko Kusaka, Shizuo Oi & Madjid Samii

Authors
  1. Laura Columbano
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  2. Wolf Luedemann
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  3. Yasuko Kusaka
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  4. Shizuo Oi
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  5. Madjid Samii
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Correspondence to Laura Columbano.

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Columbano, L., Luedemann, W., Kusaka, Y. et al. Prenatal diagnosed cyst of the quadrigeminal cistern in Aicardi syndrome. Childs Nerv Syst 25, 521–522 (2009). https://doi.org/10.1007/s00381-008-0772-8

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  • DOI: https://doi.org/10.1007/s00381-008-0772-8

Keywords

  • Aicardi syndrome
  • Fetal arachnoid cyst
  • Quadrigeminal cistern
  • Intrauterine MRI